Extrahepatic Shunt Research Articles

Membranoproliferative glomerulonephritis in a child with congenital portosystemic shunt.

Congenital portosystemic shunts (CPSS) are rare congenital vascular anomalies characterized by abnormal connections between the portal vein and systemic circulation, bypassing the liver. They can lead to complications such as recurrent encephalopathy, liver nodules, portopulmonary hypertension, and neurocognitive issues due to hyperammonemia and rarely kidney involvement. Hepatic hemodynamic changes can lead to liver nodules and hepatocellular carcinoma, particularly in extrahepatic shunts. We describe here an 11-year-old girl with type 1 intrahepatic portosystemic shunt with focal nodular hyperplasia in the liver, presenting with nephrotic syndrome that was diagnosed as membranoproliferative glomerulonephritis on kidney biopsy and that responded partially to therapy with immunosuppressants.

Efficacy and safety of precision-guided transjugular extrahepatic portosystemic shunt (TEPS) in the management of cavernous transformation of the portal vein with portal hypertension: a case series

Background and aimsPerforming a Transjugular intrahepatic portal system shunt (TIPS) in patients with portal vein cavernous transformation (CTPV) poses significant challenges. As an alternative, transjugular extrahepatic portal vein shunt (TEPS) may offer a potential solution for these patients. Nonetheless, the effectiveness and safety of TEPS remain uncertain. This case series study aimed to evaluate the efficacy and safety of TEPS in treating patients with CTPV portal hypertension complications.MethodsThe study encompassed a cohort of 22 patients diagnosed with CTPV who underwent TEPS procedures. Of these, 13 patients manifested recurrent hemorrhagic episodes subsequent to conventional therapies, 8 patients grappled with recurrent or refractory ascites, and 1 patient experienced acute bleeding but refused endoscopic treatment. Comprehensive postoperative monitoring was conducted for all patients to rigorously evaluate both the technical and clinical efficacy of the intervention, as well as long-term outcomes.ResultsThe overall procedural success rate among the 22 patients was 95.5% (21/22).During the TEPS procedure, nine patients were guided by percutaneous splenic access, three patients were guided by percutaneous hepatic access, five patients were guided by transmesenteric vein access from the abdomen, and two patients were guided by catheter marking from the hepatic artery. Additionally, guidance for three patients was facilitated by pre-existing TIPS stents. The postoperative portal pressure gradient following TEPS demonstrated a statistically significant decrease compared to preoperative values (24.95 ± 3.19 mmHg vs. 11.48 ± 1.74 mmHg, p < 0.01).Although three patients encountered perioperative complications, their conditions ameliorated following symptomatic treatment, and no procedure-related fatalities occurred. During a median follow-up period of 14 months, spanning a range of 5 to 39 months, we observed four fatalities. Specifically, one death was attributed to hepatocellular carcinoma, while the remaining three were ascribed to chronic liver failure. During the follow-up period, no instances of shunt dysfunction were observed.ConclusionsPrecision-guided TEPS appears to be a safe and efficacious intervention for the management of CTPV.

Laparoscopic treatment of congenital portosystemic shunts with portal pressure measurement and portal angiography in 36 dogs.

Laparoscopic surgery is used for canine congenital extrahepatic portosystemic shunts (CEHPSS). However, outcomes of laparoscopic surgery involving simultaneous portal vein angiography and portal pressure measurement to attenuate or completely occlude the shunt vessel in canines remain unclear. This study aimed to evaluate outcomes and complications of laparoscopic portosystemic shunt occlusion (LAPSSO) for CEHPSS. Between June 2014 and March 2021, data on dogs undergoing cellophane banding (CB) and complete occlusion of laparoscopically treated congenital extrahepatic port shunts were collected from hospital records. Cases in which complete occlusion was laparoscopically performed, or a CB was used for gradual occlusion were included. A total of 36 dogs (14 males; median age 32.5 months [range, 5-99] with median body weight, 4.2 kg [range, 1.5-7.9]) that underwent LAPSSO for CEHPSS were included. All the dogs underwent computed tomographic angiography (CTA), and data on blood and radiological examinations were collected. Shunt vessel morphology was categorized using CTA findings. Portal pressure measurements and portal angiography were performed by accessing mesenteric and splenic veins in 30 and 6 cases, respectively. The most common shunt types were spleno-phrenic shunts 16/36 (44.4%), followed by spleno-azygos 9/36 (25.0%), spleno-caval 4/36 (11.1%), right gastric-caval 6/36 (16.6%), and right gastric-caval with caudal loop shunts 1/36 (2.7%). The median portal pressure after complete occlusion was 11.5 mmHg (range, 4-16); portal pressures in the two dogs undergoing CB attenuation were 22 and 24 mmHg. The median operating time in the dogs with right (n = 25) and left (n = 11) recumbent positioning was 55 min (range, 28-120) and 54 min (range, 28-88), respectively. One dog had pneumothorax due to injury to the diaphragm. Another dog developed postoperative hypernatremia and succumbed 5 h post-procedure. Nevertheless, no other dogs exhibited signs of portal hypertension within 72 h. Blood tests and abdominal ultrasounds performed 1-2 months postoperatively revealed no residual shunts. LAPSSO, coupled with portal pressure measurement and portal angiography, was shown as safe and effective approach that facilitated successful occlusion of CEHPSS. Further large-scale prospective studies and analyses of perioperative complications are needed.

Enlargement of the hepatic artery is present in dogs with a congenital extrahepatic portosystemic shunt and is independent of shunt insertion into the systemic circulation.

The accurate diagnosis of portovascular anomalies has been facilitated by improvements in diagnostic imaging technology. In humans, hepatic arterial blood flow changes in response to the reduction in portal blood flow. The hepatic arterial buffer response characterizes an intrinsic regulatory mechanism in response to reduced portal venous blood flow, which results in hepatic arterial enlargement. At the authors' institution, enlargement of the hepatic artery has been anecdotally observed in a population of dogs with extrahepatic portosystemic shunting, consistent with previous literature that documents variability in hepatic arterial size. In this retrospective, blinded, analytical study, a hepatic artery:aorta (Ha:Ao) ratio was assessed on CT studies from 112 dogs, with (n=43) and without (n=69) an extrahepatic congenital portosystemic shunt in order to compare the hepatic artery size independent of body weight between the two populations. A significant increase in the Ha:Ao ratio was documented in dogs with an extrahepatic portosystemic shunt (EHPSS) compared with those dogs with no EHPSS independent of the location of shunt insertion into the systemic circulation (P<.001). Three cases had repeat CT after surgery, and all had Ha:Ao ratio reductions following treatment. The authors propose that this may be an additional imaging feature observed in dogs with an EHPSS.

Pulmonary arterial hypertension associated with type II Abernethy malformation in an adolescent: a case report

The article provides a case of pulmonary arterial hypertension (PAH) associated with congenital extrahepatic portocaval shunt (CEPS), or Abernethy malformation, in a 17-year-old female patient. CEPS, which remained undiagnosed for a long time, manifested with severe encephalopathy at an early age. By the age of 6 years, it was complicated by PAH, and by the age of 9 years — by hepatic focal nodular hyperplasia. In the absence of timely surgical treatment and adequate therapy of CEPS, PAH progressed. Combined dual therapy for PAH, prescribed only at age 14, was ineffective. Despite the potential for clinical and functional improvement documented with combination triple therapy at 17 years of age, the patient continued to meet criteria for a high risk of adverse events, including mortality. Two months after the initiation of triple therapy for PAH, not achieving the target hemodynamic characteristics that would reduce operative and perioperative risks lead to an attempt of surgical treatment of CEPS, which led to the patient death in the early postoperative period. The description of this case report shows the difficulties of diagnosing a rare form of portopulmonary hypertension, the modern possibilities of drug therapy for a severe, potentially curable PAH in the early stages.

Absence of Ductus Venosus: A Comparison of 2 Distinctive Fetal Autopsy Cases and Embryologic Perspectives.

In fetal circulation, oxygenated blood from the placenta flows through the umbilical vein into the ductus venosus (DV), then enters the inferior vena cava, and subsequently reaches the right atrium of the heart. The DV serves as a shunt, allowing this oxygen-rich blood to bypass the liver. The absence of the DV (ADV), also known as agenesis of the DV, is a rare congenital anomaly. Without a DV, blood from the umbilical vein must follow alternative routes to the heart. In ADV cases, blood from the umbilical vein must follow 1 of 2 primary drainage patterns: either an extrahepatic shunt or an intrahepatic shunt. This report details the antenatal ultrasound and postmortem findings of 2 fetuses diagnosed with ADV by prenatal imaging studies. The first case involved a fetus with a persistent right umbilical vein connected directly to the suprahepatic IVC, accompanied by early obliteration of the left umbilical vein and true agenesis of the DV. This fetus also had additional congenital anomalies. In contrast, the second case involved a fetus with a normal left umbilical vein that entered the liver. However, despite an ultrasound diagnosis of "absence" of the DV, a DV was present, though markedly hypoplastic and probably minimally functional or non-functional. In this case, blood from the umbilical vein likely followed an alternate intrahepatic route through the portal and hepatic veins, before reaching the heart (intrahepatic shunt). These contrasting cases emphasize the heterogeneity of vascular anomalies and embryologic origins captured by the term "ADV." Additionally, the terminology of "absence" or "agenesis" may be misleading in some purported ADV cases. Specifically, in the second case, the DV was not absent; it was markedly hypoplastic instead. This also appears to be the first reported case of a hypoplastic DV in a fetus. Both cases underscore the importance of effective collaboration and clear communication between maternal-fetal medicine specialists and pathologists.

The Portal Vein: A Comprehensive Review.

Radiologists are familiar with the appearances of a normal portal vein; variations in its anatomy are commonplace and require careful consideration due to the implications for surgery. These alterations in portal vein anatomy have characteristic appearances that are clearly depicted on CT, MR, and US images. Similarly, there are numerous congenital and acquired disorders of the portal vein that are deleterious to its function and can be diagnosed by using imaging alone. Some of these conditions have subtle imaging features, and some are conspicuous at imaging but poorly understood or underrecognized. The authors examine imaging appearances of the portal vein, first by outlining the classic and variant anatomy and then by describing each of the disorders that impact portal vein function. The imaging appearances of portal vein abnormalities discussed in this review include (a) occlusion from and differentiation between bland thrombus and tumor in vein and the changes associated with resultant hepatic artery buffer response changes, cavernous transformation of the portal vein, and portal biliopathy; (b) ascending thrombophlebitis of the portal vein (pylephlebitis); (c) portal hypertension and its causes and sequelae; (d) the newly described disease entity portosinusoidal vascular disorder; and (e) intra- and extrahepatic shunts of the portal system, both congenital and acquired (including Abernethy malformations), and the associated risks. Current understanding of the pathophysiologic processes of each of these disorders is considered to aid the approach to reporting. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

P394 PULMONARY HYPERTENSION IN SEARCH OF GROUP

Abstract Episode of haemoptysis in a 25–year–old woman with the following medical history: surgical closure of Botallo‘s duct at the age of 5 months, coeliac disease, growth retardation, polycystic ovary, visus alterations. In the emergency room CT angiography of the chest showed the presence of subpleural parenchymal consolidation in the right lower lobe, with associated ground–glass opacities, cardiomegaly, severe pulmonary artery dilatation and hepatomegaly with intrathoracic involvement by a hernia of Morgagni on the right side. A porto–systemic shunt with an intrahepatic course was also noted in the duct at the VIII segment with an autonomic outlet in the right atrium. The patient was transferred to the Internal Medicine ward. Objective examination revealed an elf facies with hypertelorism, short stature, bradypsychism. On interview she reported dyspnoea on light exertion and frequent lipohymic episodes. Haematochemical examinations showed increased of bilirubinemia mainly indirect, Transaminases and of NTproBNP and normal ammonium levels. Blood gas analysis showed a tendency to respiratory alkalosis with oxyaemia at lower limits. Because of the suspicion of a syndromic picture, a genetic examination was performed. Cardiological evaluation showed a high ultrasound probability of pulmonary hypertension (PH). Transferred to cardiology, catheterisation confirmed pulmonary arterial hypertension (mPAP 64mmHg) precapillary (wedge 4mmHg) with high PVR (11 Wood Units) not responsive to nitric oxide. In addition, there was a superior vena cava protruding into the coronary sinus and an extrahepatic shunt with an isolated outlet in the RA without gradient. The hepatobiliary surgery consultation concluded for Abernethy syndrome, which is a rare congenital syndrome characterised by an extrahepatic portosystemic shunt in which the blood from the splanchnic and lienal circulation bypasses the portal vein and the liver, draining into the systemic circulation via an abnormal conduit. PH is a rare complication of this syndrome, maintained by hyperafflux, vasoactive mediators and the formation of microthrombi and intimal fibrosis in the pulmonary circulation. An HR–CT excluded venulo–capillary plexiform disease. Macitentan 10 mg/day was introduced, then continued at home for the benefit shown and follow–up at the Pulmonary Work Group was initiated. This PH, due to its association with an extremely rare pathology, required the collaboration of a diverse group of specialists.

Opioid‐free anaesthesia for surgical attenuation of a congenital extrahepatic portosystemic shunt in a dog

AbstractAnimals with impaired hepatic metabolism may experience reduced effect from some anaesthetic drugs and are more prone to developing unwanted side effects. Due to reduced blood flow to the liver, it is important to carefully select anaesthetic drugs in patients with extrahepatic shunt. We present the case in which a dog was submitted for surgical attenuation of congenital extrahepatic portosystemic shunt through placement of an ameroid constrictor. A multimodal regimen was designed in order to provide an opioid‐free anaesthesia, relying on analgesia through a locoregional quadratus lumborum block with bupivacaine. Dexmedetomidine was administered intramuscularly as pre‐anaesthetic medication, propofol was used for the induction and anaesthesia was maintained with isoflurane. The present case describes the first case in which an extrahepatic portosystemic shunt was surgically treated using an opioid‐free anaesthesia in a dog.

Prediction of outcome after ligation or thin film banding of extrahepatic shunts, based on plasma albumin concentration and hematologic expression of 8 target genes in 85 dogs.

In dogs with a congenital extrahepatic portosystemic shunt (EHPSS), outcome after surgical attenuation is difficult to predict. Develop a minimally invasive test to predict outcome after surgical EHPSS attenuation and establish risk factors for postattenuation seizures (PAS). Eighty-five client-owned dogs referred for surgical attenuation of a single EHPSS. mRNA expression of 8 genes was measured in preoperatively collected venous blood samples. Outcome was determined at a median of 92 days (range, 26-208) postoperatively by evaluating clinical performance, blood test results and abdominal ultrasonography. Multivariable logistic regression was used to construct models predicting clinical and complete recovery. The associations between putative predictors and PAS were studied using univariable analyses. Five of 85 dogs developed PAS. Risk factors were age, white blood cell (WBC) count and expression of hepatocyte growth factor activator and LysM and putative peptidoglycan-binding domain-containing protein 2. Clinical recovery was observed in 72 of 85 dogs and complete recovery in 51 of 80 dogs (median follow-up, 92 days). The model predicting clinical recovery included albumin, WBC count, and methionine adenosyltransferase 2 alpha (MAT2α) expression, whereas the model predicting complete recovery included albumin, and connective tissue growth factor precursor and MAT2α expression. The areas under the receiver operating characteristic curves were 0.886 (95% confidence interval [CI]: 0.783, 0.990) and 0.794 (95% CI: 0.686, 0.902), respectively. Two models were constructed for predicting outcome after EHPSS attenuation using venous blood samples. The model predicting clinical recovery showed the best diagnostic properties. Clinical application requires further validation.

Congenital Portosystemic Shunts: Variable Clinical Presentations Requiring a Tailored Endovascular or Surgical Approach.

Congenital portosystemic shunts (CPSS) are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation. These shunts allow intestinal blood to reach the systemic circulation directly, and if persistent or large, may lead to long-term complications. CPSS can have a variety of clinical presentations that depend on the substrate that is bypassing hepatic metabolism or the degree of hypoperfusion of the liver. Many intrahepatic shunts spontaneously close by 1 year of age, but extrahepatic and persistent intrahepatic shunts require intervention by a single session or staged closure with a multidisciplinary approach. Early detection and appropriate management are important for a good prognosis. The aim of this case series is to describe the varied clinical presentations, treatment approaches, and outcomes of 5 children with CPSS at our institution. Management of these patients should involve a multidisciplinary team, including interventional radiology, surgery, hepatology, and other medical services as the patient's clinical presentation warrants. Regardless of clinical presentation, if a CPSS persists past 1-2 years of age, closure is recommended.

Congenital portosystemic shunts: surgical treatment experience

Congenital porto-caval shunts are rare and may have a different morphological structure (intra- and extrahepatic shunts, with or without portal blood flow). The main method of treating patients with this pathology is endovascular shunt occlusion. However, in some cases, this method is ineffective.&#x0D; The article contains a description of six clinical examples of surgical treatment of congenital porto-systemic shunts in children. In the diagnosis of congenital portosystemic shunts, the leading role belongs to Doppler ultrasound, multislice computed tomography, and angiography. The indication for surgical treatment was the anatomical features of the shunt, which makes endovascular occlusion technically impossible. In one observation a wide Arantian duct was diagnosed, its open ligation was performed. In another case, the portal vein emptied directly into an aneurysmal dilatation, performed reconstructive plastic surgery on the vessels of the portal vein. In the next observation, a pronounced retrograde blood flow was determined along the dilated inferior mesenteric vein, blood was discharged through the sacral plexus into the internal iliac vein. The left internal iliac vein was isolated and ligated, the dysplastic inferior mesenteric vein was ligated and partially removed. In 2 patients, the portal vein flowed directly into the inferior vena cava in the area of aneurysmal expansion; an operation was performed open ligation of the shunt. In one observation, a deep hypoplasia of the intrahepatic branches of the portal vein was diagnosed, and therefore the restoration of portal blood flow after the closure of the shunt is impossible. The child was sent to decide on a liver transplant.&#x0D; Each case of congenital porto-caval shunts is unique. The surgeon determines the tactics directly during the operation, depending on the morphological structure of the organs, since the preoperative examination does not always give an unambiguous idea.

Attenuation of right gastrocaval extrahepatic shunt with cellophane band in a dog: Case report

Congenital extrahepatic portosystemic shunt is a vascular anomaly that consists in the communication between the portal and systemic circulation, causing substances normally metabolized and excreted by the liver to be diverted directly to the systemic circulation and triggering changes in neurological, gastrointestinal and urinary systems. Diagnosis is through imaging tests, such as ultrasound and computed tomography. For patient stabilization, initial clinical treatment is highly indicated, especially for those who manifest signs of hepatic encephalopathy, and the definitive treatment is gradual surgical attenuation. Currently, the most indicated techniques for shunt occlusion are the ameroid constrictor ring and the cellophane band, which have good long-term results. The prognosis of patients undergoing the surgical procedure varies according to the general condition of the patient at the time of diagnosis and the complications that may occur in the trans and postoperative periods, such as portal hypertension, seizures, hypoglycemia and failure to close the shunt. This report aims to describe a case of surgical attenuation of a right extrahepatic gastrocaval shunt using the cellophane band technique. The canine patient, pug, aged eight months, was treated at the Veterinary Hospital of the Federal University of Paraná, presenting neurological clinical signs and a previous diagnosis of portosystemic shunt. To determine the origin of the anomalous vessel and optimize surgical planning, computed angiotomography was indicated. Clinical treatment was instituted and, after 15 days, surgical attenuation of the shunt was performed using the cellophane band fixed with titanium clips. The patient showed signs of portal hypertension, ascites and postoperative hypoglycemia, which were treated during hospitalization. After 15 days of hospital discharge, the patient returned to the Veterinary Hospital with good general condition, absence of ascites and neurological signs and no significant changes were observed in laboratory tests, demonstrating the effectiveness of the chosen surgical technique.

Perinatal Outcomes According to the Type of Prenatally Diagnosed Umbilical-Portal-Systemic Venous Shunt

Background: The abnormal course of the umbilical vein and the absence or displacement of the portal vein or ductus venosus may suggest the presence of other shunts. In recent years, an in utero classification of umbilical-portal-systemic venous shunts (UPSVS) has been introduced. This study aims to report our experience with the prenatal diagnosis of UPSVS and to evaluate the perinatal outcomes of fetuses with different types of UPSVS. Methods: This was a retrospective study of fetuses prenatally diagnosed with UPSVS between January 2005 and September 2020 at Asan Medical Center. UPSVS was prenatally classified into three types according to the anatomical origin of the shunt: type I, umbilical-systemic shunt; type II, ductus venosus-systemic shunt; and type III, portal-systemic shunt. Type III was further divided into two subtypes: type IIIa, intrahepatic shunt; type IIIb, extrahepatic shunt. Postnatal ultrasonography or abdominal computed tomography (CT) was performed to confirm UPSVS. Results: Out of 18 fetuses prenatally diagnosed with UPSVS, four were excluded. All four patients were confirmed to have portal vein variations without a shunt. The median gestational age at the diagnosis of the remaining 14 fetuses was 30.3 weeks (range, 23.1–37.3). The median gestational age at birth was 37.2 weeks (range, 31.0–39.3), and the median birth weight was 2105 g (range, 1350–2870), including nine (64%) who had body weights below the 10th percentile. Eleven patients (79%) had combined anomalies; cardiomegaly and fetal hydrops were observed in eight (57%) and two (14%) patients, respectively. Among the 11 patients who underwent chromosomal analysis, three had abnormal results. Two infants with abnormal karyotypes died. The most common type was type I (8, 57%), followed by type IIIa (4, 29%), type II (1, 7%), and type IIIb (1, 7%). In type I, all but one patient had structural anomalies; five (63%) showed cardiomegaly and five (63%) were associated with growth restriction. Nevertheless, half of the patients survived without major anomalies. In type IIIa, three (75%) patients were associated with growth restriction, but they showed catch-up growth after birth. All patients had mild transient hyperammonemia. One type II patient was associated with cardiomegaly, fetal hydrops, and an abnormal karyotype. One type IIIb patient had multiple anomalies and an abnormal karyotype. No surgical correction was required because of the UPSVS. Conclusions: Prenatal diagnosis of UPSVS is feasible. When an abnormal course of the umbilical or portal vein is detected on the transverse abdominal view, UPSVS should be suspected. UPSVS is commonly associated with structural anomalies, chromosomal anomalies, growth restriction, and cardiomegaly. UPSVS without associated anomalies may have a favorable prognosis. Our study can help predict the prognosis of UPSVS and assist in prenatal counseling.

S3320 An Intrahepatic Portosystemic Shunt Masquerading as a Tumor

Introduction: Portosystemic shunts can be congenital or acquired and are divided into extrahepatic and intrahepatic shunts. Intrahepatic portosystemic venous shunts (IPSVSs) are abnormal intrahepatic connections that occur between branches of the portal vein (PV) and hepatic veins (HV) or IVC. Patients with IPSVSs can be symptomatic or asymptomatic as most of the cases are detected incidentally on imaging. However, they can potentially lead to long term complications by allowing bypass of mesenteric venous return into systemic circulation without going through the liver. We herein describe the case of a 58-year-old woman who was incidentally found to have an IPSVS on imaging for which she was recommended periodic surveillance imaging. Case Description/Methods: A 58-year-old woman was undergoing workup for weight loss and was noted to have a 2.3 x 2.0 cm hyperdense nodular lesion in the posterior segment of the right hepatic lobe suspicious for hemangioma or a hypervascular neoplasm on CT abdomen pelvis. She was referred to the hepatobiliary clinic and a CTA abdomen pelvis was then performed and showed a lobulated 2.2 x 2.1 x 2.9 cm lesion in segment 6 consistent with an intrahepatic portosystemic shunt (figure 1). The patient was otherwise asymptomatic and therefore was advised to continue with periodic imaging surveillance. Discussion: IPSVSs are divided into 4 types depending on their morphology. In type 1, a single large vessel connects the PV or its right branch to the IVC. Type 2 involves a localized peripheral shunt within one hepatic segment that has one or more communications between peripheral branches of the PV and HV. In type 3, an aneurysmal connection exists between PV and HV. And finally, multiple communications between PV and HV in multiple lobes are present in type 4. In patients without a history of liver disease or trauma, the origin of the IPSVS is presumed to be congenital. CT scan, MRI and even color Doppler sonograms should provide visualizations of the feeding portal vein and draining hepatic vein. For symptomatic IPSVSs, endovascular closure is the standard of care. However, no consensus exists for asymptomatic cases and surveillance is an appropriate option in asymptomatic patients.Figure 1.: Axial CTA abdomen venous phase showing aneurysmal communication (red arrow) between right portal vein (yellow arrow) and middle hepatic vein (green arrow) in segment 6 of the liver.

Abernethy malformation

A 59-years-old woman presented to the emergency department with sudden onset of intermittent hematemesis and melena. The patient had a history of stage IV cervical cancer, sigmoid colostomy (because of rectovaginal fistula). A complete blood count showed normocytic anemia (79 g/l) and other laboratory evaluation showed the elevation of ammonia (38 µmol/l). The total bilirubin, transaminase and creatinine level were normal. Upper endoscopy revealed hemorrhage of esophageal varices that was stopped by local treatment with the combinations of spraying hemostatic and sclerotherapy (Figure 1). The CT-scan revealed no cirrhosis and liver lesion but did 10 show the absence of the portal vein with a complete extrahepatic shunt of the portal blood, considered Abernethy Malformation Ib (Figure 2,3), determined to be the possible reason of esophageal varices and megalosplenia. The old patient suffered from first-onset gastrointestinal bleeding and was diagnosed the only curative treatment is liver transplantation. This patient was stopped bleeding and transferred to cancer center for tumor therapy.

A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME

BackgroundCongenital portosystemic shunts (CPSS) are rare vascular malformations that result in blood bypassing the liver and re-entering the systemic circulation unfiltered. The outcomes of CPSS diagnosed prenatally are unknown. Previously published data is related to patients diagnosed after symptom appearance, including hepatic encephalopathy, liver tumors and portal hypertension.AimsTo describe a cohort of prenatally diagnosed children with CPSS and report on the natural history and outcomes.MethodsThe study was conducted between 2006 and 2019. Prenatal diagnosis was performed during routine prenatal ultrasound. Patients were followed by the pediatric gastroenterologists’ unit.Results32 patients were identified; 28 patients with intrahepatic shunts and four patients with extrahepatic shunts. During follow up, Failure to Thrive (FTT) was observed in three of the patients with one shunt (16.7%), and five of the patients with two shunts or more (55.6%). The difference is significant (p- value=0.037). 24 patients with intrahepatic shunts had their shunts closed spontaneously. Median time for closure of the shunt was seven months (IQR 2–14 months, range 0–35 months). No predicting factor was detected for closure of the shunt before the age of one year. All extrahepatic shunts required surgical closure.ConclusionsCongenital intrahepatic shunts usually close spontaneously and do not need intervention. All patients in our prenatally diagnosed cohort survived with limited to no sequelae.Funding AgenciesNone

A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report

BackgroundCongenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment.Case presentationWe describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months.ConclusionsPortal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.

Persistent hypercobalaminemia three months after successful gradual attenuation of extrahepatic shunts in dogs: a prospective cohort study

BackgroundDeficiencies in vitamin A and D and disorders in the vitamin B complex are often present in people with chronic liver diseases. So far, the serum concentrations of these vitamins have not yet been studied in dogs with congenital extrahepatic portosystemic shunts (EHPSS), who also have some degree of liver dysfunction. The objective was to assess serum vitamin concentrations in dogs with EHPSS from diagnosis to complete closure. A prospective cohort study was performed using ten client-owned dogs with EHPSS, closed after gradual surgical attenuation. Serum concentrations of vitamin A, 25-hydroxyvitamin D, folic acid, cobalamin and methylmalonic acid (MMA) were measured at diagnosis prior to institution of medical therapy, prior to surgery, and three months after gradual attenuation and complete closure of the EHPSS.ResultsAt diagnosis, median serum concentrations of vitamin A, 25-hydroxyvitamin D and folic acid were 18.2 μg/dL (8.8 - 79.5 μg/dL), 51.8 ng/mL (19.4 - 109.0 ng/mL), and 8.1 μg/L (5.2 - 14.5 μg/L), respectively, which increased significantly postoperatively (88.3 μg/dL (51.6 - 182.2 μg/dL, P=0.005), 89.6 ng/mL (49.3 - >150.0 ng/mL, P =0.005), and 14.8 μg/L (11.5 - 17.7 μg/L, P <0.001), respectively). Median serum cobalamin concentrations were 735.5 ng/L (470 - 1388 ng/L) at diagnosis and did not significantly decrease postoperatively (P =0.122). Both at diagnosis and three months postoperatively 7/10 dogs had hypercobalaminemia.ConclusionsSerum concentrations of vitamin A, 25-hydroxyvitamin D and folic acid significantly increase after surgical attenuation. Nevertheless, persistent hypercobalaminemia is suggestive of ongoing liver dysfunction, despite successful surgery.

Postattenuation neurologic signs after surgical attenuation of congenital portosystemic shunts in dogs: A review

The development of postattenuation neurologic signs (PANS) is a poorly understood and potentially devastating complication after surgical attenuation of congenital portosystemic shunts in dogs. Postattenuation neurologic signs include seizures but also more subtle neurologic signs such as depression, behavioral changes, tremors, and twitching. They most commonly occur within 7 days postoperatively and are typically unrelated to hyperammonemia, hypoglycemia, or electrolyte disturbances. This narrative review summarizes the findings of 50 publications from 1988-2020 that report occurrence of PANS. While most published reports included only dogs affected by postattenuation seizures (PAS), others included dogs with any form of PANS. Overall, PANS (including PAS) affected 1.6%-27.3% of dogs, whereas incidence of PAS ranged from 0%-18.2%. The etiology of PANS remains unknown; however, several theories have been proposed. Risk factors include preoperative hepatic encephalopathy, increasing age, and possibly certain breeds and extrahepatic shunt morphology. There is increasing evidence that prophylactic antiepileptic drugs do not prevent PANS. Treatment is centered around controlling neurologic signs with antiepileptic drugs and providing supportive intensive care. The 30-day survival rate in studies that included a minimum of four dogs affected by PANS was 0%-100% (median, 50.0%) and 0%-75.0% (median, 37.5%) for those with PAS. Mortality associated with PANS was typically related to occurrence of generalized seizure activity. Prognostic factors positively associated with short-term survival included having a history of preoperative seizures and development of focal seizures only. If affected dogs survived to discharge, survival for several years was possible, and the majority of neurologic signs manifested as part of the phenomenon of PANS appeared to resolve.